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Diagnosis and Treatment > Signs and Symptoms

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Sharmin Jahan Department of Endocrinology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh

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M A Hasanat Department of Endocrinology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh

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Tahseen Mahmood Department of Endocrinology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh

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Shahed Morshed Department of Endocrinology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh

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Raziul Haq Department of Neurosurgery, Dhaka Medical College and Hospital (DMCH), Dhaka, Bangladesh

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Md Fariduddin Department of Endocrinology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh

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Summary

Silent corticotroph adenoma (SCA) is an unusual type of nonfunctioning pituitary adenoma (NFA) that is silent both clinically and biochemically and can only be recognized by positive immunostaining for ACTH. Under rare circumstances, it can transform into hormonally active disease presenting with severe Cushing syndrome. It might often produce diagnostic dilemma with difficult management issue if not thoroughly investigated and subtyped accordingly following surgery. Here, we present a 21-year-old male who initially underwent pituitary adenomectomy for presumed NFA with compressive symptoms. However, he developed recurrent and invasive macroadenoma with severe clinical as well as biochemical hypercortisolism during post-surgical follow-up. Repeat pituitary surgery was carried out urgently as there was significant optic chiasmal compression. Immunohistochemical analysis of the tumor tissue obtained on repeat surgery proved it to be an aggressive corticotroph adenoma. Though not cured, he showed marked clinical and biochemical improvement in the immediate postoperative period. Anticipating recurrence from the residual tumor, we referred him for cyber knife radio surgery.

Learning points:

  • Pituitary NFA commonly present with compressive symptoms such as headache and blurred vision.

  • Post-surgical development of Cushing syndrome in such a case could be either drug induced or endogenous.

  • In the presence of recurrent pituitary tumor, ACTH-dependent Cushing syndrome indicates CD.

  • Rarely a SCA presenting initially as NFA can transform into an active corticotroph adenoma.

  • Immunohistochemical marker for ACTH in the resected tumor confirms the diagnosis.

Taxonomy:
Clinical Overview, Gland/Organ, Pituitary, Topic, Pituitary, Hormone, ACTH, Cortisol, FSH, Prolactin, Testosterone, Condition/ Syndrome, Corticotrophic adenoma, Cushing's disease, Cushing's syndrome, Hypogonadotrophic hypogonadism, Non-functioning pituitary adenoma, Patient Demographics, Age, Adolescent/young adult, Gender, Male, Ethnicity, Asian - Bangladeshi, Country of Treatment, Bangladesh, Diagnosis and Treatment, Signs and Symptoms, Acanthosis nigricans, Central obesity, Dyslipidaemia, Facial plethora, Facies - moon, Hypogonadism, Leukocytosis, Obesity, Striae, Supraclavicular fat pads, Visual disturbance, Visual field defect, Weight gain, Investigation, ACTH, Blood pressure, Cortisol (plasma), Cortisol, free (24-hour urine), CT scan, Dexamethasone suppression (high dose), Dexamethasone suppression (low dose), FSH, Histopathology, Immunohistochemistry, Immunostaining, Liver function, MRI, Prolactin, Testosterone, Intervention, Gamma knife radiosurgery, Radiotherapy, Resection of tumour, Transsphenoidal surgery, Medication, Dexamethasone, Glucocorticoids, Related Disciplines, Ophthalmology, Pathology, Surgery, Publication Details, Case Report Type, Error in diagnosis/pitfalls and caveats
DOI:
https://doi.org/10.1530/EDM-19-0046
Volume/Issue:
Volume 2019: Issue 1
Open access
Lima Lawrence Departments of Endocrinology, Diabetes & Metabolism, Cleveland Clinic, Cleveland, Ohio, USA

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Peng Zhang Departments of Pulmonary Medicine & Critical Care, Cleveland Clinic, Cleveland, Ohio, USA

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Humberto Choi Departments of Pulmonary Medicine & Critical Care, Cleveland Clinic, Cleveland, Ohio, USA

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Usman Ahmad Departments of Thoracic and Cardiovascular Surgery, Cleveland Clinic, Cleveland, Ohio, USA

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Valeria Arrossi Departments of Anatomic Pathology, Cleveland Clinic, Cleveland, Ohio, USA

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Andrei Purysko Departments of Diagnostic Radiology, Cleveland Clinic, Cleveland, Ohio, USA

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Vinni Makin Departments of Endocrinology, Diabetes & Metabolism, Cleveland Clinic, Cleveland, Ohio, USA

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Summary

Ectopic adrenocorticotropic hormone (ACTH) production leading to ectopic ACTH syndrome accounts for a small proportion of all Cushing’s syndrome (CS) cases. Thymic neuroendocrine tumors are rare neoplasms that may secrete ACTH leading to rapid development of hypercortisolism causing electrolyte and metabolic abnormalities, uncontrolled hypertension and an increased risk for opportunistic infections. We present a unique case of a patient who presented with a mediastinal mass, revealed to be an ACTH-secreting thymic neuroendocrine tumor (NET) causing ectopic CS. As the diagnosis of CS from ectopic ACTH syndrome (EAS) remains challenging, we emphasize the necessity for high clinical suspicion in the appropriate setting, concordance between biochemical, imaging and pathology findings, along with continued vigilant monitoring for recurrence after definitive treatment.

Learning points:

  • Functional thymic neuroendocrine tumors are exceedingly rare.

  • Ectopic Cushing’s syndrome secondary to thymic neuroendocrine tumors secreting ACTH present with features of hypercortisolism including electrolyte and metabolic abnormalities, uncontrolled hypertension and hyperglycemia, and opportunistic infections.

  • The ability to undergo surgery and completeness of resection are the strongest prognostic factors for improved overall survival; however, the recurrence rate remains high.

  • A high degree of initial clinical suspicion followed by vigilant monitoring is required for patients with this challenging disease.

Taxonomy:
Clinical Overview, Gland/Organ, Adrenal, Pituitary, Thymus, Topic, Adrenal, Hormone, ACTH, Cortisol, Condition/ Syndrome, Bilateral adrenal hyperplasia, Cushing's syndrome, Ectopic ACTH syndrome, Ectopic Cushing's syndrome, Hypokalaemia, Lymphadenopathy, Neuroendocrine tumour, Rhabdomyolysis, Patient Demographics, Age, Adult, Gender, Male, Ethnicity, White, Country of Treatment, United Kingdom, Diagnosis and Treatment, Signs and Symptoms, Buffalo hump, Chest pain, Chest pain (pleuritic), Dyspnoea, Facial fullness, Facial plethora, Hypercortisolaemia, Hyperglycaemia, Hyperpigmentation, Hypertension, Hypokalaemia, Hypotension, Leukocytosis, Lymphadenitis, Metabolic alkalosis, Myasthaenia, Obesity, Photosensitivity, Rhabdomyolysis, Supraclavicular fat pads, Syncope, Weight gain, Investigation, ACTH, ACTH stimulation, Bicarbonate, Biopsy, Blood pressure, Chromogranin A, Cortisol, Cortisol, free (24-hour urine), Creatine kinase, CT scan, Dexamethasone suppression, Glucose (blood), Histopathology, Immunohistochemistry, MRI, PET scan, Potassium, Synaptophysin, White blood cell count, X-ray, Intervention, Resection of tumour, Medication, Dexamethasone, Glucocorticoids, Ketoconazole, Steroids, Publication Details, Case Report Type, Unique/unexpected symptoms or presentations of a disease
DOI:
https://doi.org/10.1530/EDM-19-0002
Volume/Issue:
Volume 2019: Issue 1
Open access